Two cases of subependymoma in the cervical spinal cord associated with stigmata of neurofibromatosis are reported. Magnetic resonance (MR) imaging showed one tumor with a sharp margin, which was well-demarcated intraoperatively and was totally removed. MR imaging showed the other tumor with an irregular margin, which was partly invasive at operation. Subependymomas are rare in the spinal cord and these are the first reported associations with neurofibromatosis.